Amyotrophic lateral sclerosis (ALS) is a degenerative condition of the nerve cells of the spinal cord and brain. It is a rapid and insidious killer—patients go from initial symptoms to death in typically just over three years. The average sufferer’s neural pathways eventually deteriorate until the point of respiratory failure.
In 2014, name recognition for the disease reached an all-time high, and fundraising efforts are breaking records, but the average person still knows very little about ALS.
1.The Limits Of The Ice Bucket Challenge
Until a few months ago, a good number of people hadn’t even heard of ALS. That all changed when the Ice Bucket Challenge went viral in the summer of 2014. Exactly who originated the challenge seems unknown, but versions with cold water can be traced to 2013. As of this writing, the ALS Association has raised over $100 million, a 3,500-percent increase in donations over the same period last year.
2.ALS And Athletes
Athletes are at a higher risk for ALS than the general population. Indeed, ALS is often referred to by its more colloquial name, Lou Gehrig’s disease, after the legendary first baseman for the New York Yankees.
For over 50 years, Gehrig held the record for the most consecutive games played, an accomplishment that earned him the nickname “The Iron Horse.” Yet even his stern constitution couldn’t save him from ALS, and he said goodbye to his fans at Yankee Stadium on July 4, 1939. In his famous farewell speech, he said that despite his grim diagnosis, he felt like “the luckiest man on the face of the Earth.” Lou held on for only two more years, dying of ALS on June 2, 1941 at 37 years of age.
This phenomenon is not limited to just the United States. Italian soccer players have also exhibited a strong propensity toward developing ALS—six times that of the average Italian. According to one theory, this is due to the average player’s frequent contact with ticks in the grass.
3.An Orphan Disease
ALS affects only 2 out of every 100,000 people. This makes it a so-called orphan disease, one that pharmaceutical companies have yet to adopt as a major research subject because they’ll make no real money from it. More prevalent conditions, from depression to erectile dysfunction, are the ones that bring in billions in profits.
As a result, few treatment options exist. There is only a single drug on the market for ALS, a substance called Riluzole, approved by the FDA back in1995. The drug blocks toxin-sensitive sodium channels in neurons, slowing down the disease’s progression. Riluzole costs approximately $10 a tablet, and its effectiveness is debatable, usually extending the life of ALS sufferers by only two or three months.
4.The Mystery Of Stephen Hawking’s Survival
By the far the most famous living sufferer of ALS, Stephen Hawking is a million-to-one anomaly. The brilliant theoretical physicist was diagnosed when he was just 21 years old and was told that he had little time left. Over half a century later, he is still alive, utterly defying the odds. In the years since his diagnosis, he has made countless contributions toward human understanding of the universe.
Hawking’s doctors do not discuss the specifics of his condition, so we don’t know precisely by what mechanism he continues to soldier on. ALS affects the motor neurons that control the diaphragm and the muscles involved in swallowing, so sufferers usually die from respiratory failure or suffer malnutrition and dehydration. According to Leo McCluskey, medical director of the ALS Center at the University of Pennsylvania, “If you don’t have these two things, you could potentially live for a long time—even though you’re getting worse.
5.The Paralyzed Screenwriter
Stephen Hawking isn’t the only famous person with ALS attempting to make the best of a bad situation. Scott Lew is a screenwriter with several works under his belt, including 2013’s Sexy Evil Genius. He is in the latter stages of the disease and can only move a few muscles in his face and eyes. It takes assistants four hours just to get him out of bed and ready to work for the day.
Scott was the subject of the short documentary Jujitsu-ing Reality, a powerful and inspiring look at continuing to be productive while living with ALS. It has screened in film festivals around the world.
Like Hawking, Lew has defied the odds, living with the disease for 11 years as of 2014. He maintains an active blog full of poetry, video clips, and private musings. In one essay, titled “A Good Night With ALS,” he describes his condition as “being buried alive in your own body” but goes on to describe the beauty of life, claiming: “Every day is a chance to have fun and be creative.”
ALS is genetic in 5–10 percent of cases, but in most instances, its cause is a mystery. There are cases of married couples where both partners come down with the disease, which is odd because ALS is not contagious. Researchers believe these cases are likely just coincidences, but no one really knows for sure. In some instances, one spouse was not diagnosed until years after the partner’s death.
At least 20 known cases of conjugal ALS have cropped up around the world, including one each from India and Spain, two from Brazil, three from the United States, four from Italy, and nine from France. All the couples were together for at least a decade before their diagnosis.
Although athletes have shown a significantly higher rate of ALS than people in other fields, it affects people in a wide variety of professions, from musicians like Leadbelly and Charles Mingus to politicians like former senator Jacob Javits. It is even rumored that Chairman Mao Zedong died of ALS. But perhaps the most ironic victim of the disease was Dr. Richard Olney.
Olney was a pioneer in the study of ALS, establishing the ALS Treatment and Research Center at the University of California, San Francisco. Then in 2004, Olney’s physical state began to decline. By 2005, one of the world’s foremost experts in ALS was certain that he had the disease himself.
Olney lived for eight years with ALS, only experiencing the most catastrophic symptoms toward the very end. Even up to his last days, he continued research and worked on papers with the assistance of his son. He also did interviews to direct as much attention to the cause and his research center as possible. Olney died on January 27, 2012, aged 64.
Though the medical community does not recognize a set cause of ALS, intriguing patterns indicate that environmental factors play a role. Strange geographic clusters of the disease have popped up. Three people who wereall Ashkenazi Jews developed ALS in Montreal within an 18-month timespan. They all lived in one apartment building. In another case, four people in rural South Dakota (three of whom lived within a couple miles of each other) developed ALS within a decade.
Soldiers returning home from serving in the 1991 Gulf War have also demonstrated an unusually high degree of ALS. These soldiers could have come into contact with any number of chemicals during the war, including nerve agents, pesticides, and radioactive substances. Certain countries, such as Mexico, Italy, and Poland, report lower-than-normal levels of ALS.